2cui

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Template:STRUCTURE 2cui

Contents 1 Solution structure of the 31st fibronectin type III domain of the human tenascin X 2 Disease 3 Function 4 About this Structure 5 Reference

Solution structure of the 31st fibronectin type III domain of the human tenascin X

Disease

[TENX_HUMAN] Defects in TNXB are the cause of tenascin-X deficiency (TNXD) [MIM:606408]. TNXD leads to an Ehlers-Danlos-like syndrome characterized by hyperextensible skin, hypermobile joints, and tissue fragility. Tenascin-X-deficient patients, however, lack atrophic scars, a major diagnostic criteria for classic Ehlers-Danlos. Delayed wound healing, which is also common in classic EDS, is only present in a subset of patients.

Function

[TENX_HUMAN] Appears to mediate interactions between cells and the extracellular matrix. Substrate-adhesion molecule that appears to inhibit cell migration. Accelerates collagen fibril formation. May play a role in supporting the growth of epithelial tumors.^[1]

About this Structure

2cui is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

1. ↑ Egging D, van den Berkmortel F, Taylor G, Bristow J, Schalkwijk J. Interactions of human tenascin-X domains with dermal extracellular matrix molecules. Arch Dermatol Res. 2007 Jan;298(8):389-96. Epub 2006 Oct 11. PMID:17033827 doi:10.1007/s00403-006-0706-9